From the Aix Marseille Univ (Z.P., A.M., C.B., A.R., S.D., P.D., J. Pelletier, B.A.), APHM, Service de Neurologie, CRMBM UMR 7339, CNRS, Marseille; Hôpital Pierre Wertheimer (J. Pique, R.M.), Hospices Civils de Lyon, France; APHP (C.P., E.M.), Hôpital de la Pitié Salpêtrière, Paris; Hôpital Universitaire de Strasbourg (N.C.), Strasbourg; CHU Montpellier (X.A.), Montpellier; University of Lille (H.Z.); Fondation Rothschild (R.D.), Paris; and CHU Toulouse (J.C.), France.
Neurol Neuroimmunol Neuroinflamm. 2022 Apr 26;9(4). doi: 10.1212/NXI.0000000000001167. Print 2022 Jul.
Pure relapsing short myelitis with clinical and paraclinical features suggesting multiple sclerosis (MS) has been described recently. Here, we evaluated the existence of this potential new form of MS by retrospectively searching for similar cases in the databases of the French tertiary MS centers.
Patients were included based on the present criteria: at least 2 short (<3 vertebral segments) myelitis episodes; minimum follow-up of 3 years; no MS-like brain lesion during all the follow-up; tested negative for both anti-myelin oligodendrocyte glycoprotein and anti-aquaporin 4 antibodies in serum; presence of oligoclonal bands in CSF; and comprehensive workup to exclude alternative diagnoses.
Eighteen patients fulfilled all criteria. The sex ratio (females/males) was 5/1; the median (range) age at first relapse was 35.5 (25-54) years, the disease duration was 80.5 (50-308) months, and the annualized relapse rate was 0.36 (0.1-0.5). The median (range) number of relapses per patient was 2 (2-5), and the median (range) Expanded Disability Status Scale score at last follow-up was 1 (0-7.5). In CSF, the median (range) protein level was 0.34 g/L (0.18-0.77), and the median (range) number of mononuclear cells was 3 (0-28). Spinal cord MRI demonstrated a median (range) number of 2 (1-5) lesions per examination and 3 [1-7] on the last examination. Fifty-five percent of lesions involved the cervical levels. Secondary progressive evolution occurred in 3 of 18 (17%) patients.
Pure spinal MS could be a rare entity in the MS disease spectrum. However, the existence of a distinct entity in the inflammatory CNS disorders cannot be excluded.
近期描述了一种具有临床和辅助检查提示多发性硬化症（MS）特征的单纯复发性短节段脊髓炎。在此，我们通过检索法国三级 MS 中心的数据库，回顾性地寻找类似病例，以评估这种潜在的 MS 新形式是否存在。
根据目前的标准纳入患者：至少有 2 次（<3 个椎体节段）短节段脊髓炎发作；至少 3 年的随访；整个随访期间无脑内 MS 样病灶；血清抗髓鞘少突胶质细胞糖蛋白和抗水通道蛋白 4 抗体均为阴性；CSF 中存在寡克隆带；并进行全面检查以排除其他诊断。
18 例患者均符合所有标准。男女比例为 5/1；首次复发时的中位（范围）年龄为 35.5（25-54）岁，疾病病程为 80.5（50-308）个月，年复发率为 0.36（0.1-0.5）。每位患者的中位（范围）复发次数为 2（2-5）次，末次随访时的扩展残疾状况量表评分的中位数（范围）为 1（0-7.5）。CSF 中，中位（范围）蛋白水平为 0.34 g/L（0.18-0.77），中位（范围）单核细胞数为 3（0-28）。脊髓 MRI 显示每次检查的中位数（范围）病变数为 2（1-5），最后一次检查为 3 [1-7]。55%的病变累及颈段。18 例中有 3 例（17%）患者发生继发性进展。
单纯脊髓 MS 可能是 MS 疾病谱中的一种罕见实体。然而，不能排除 CNS 炎症性疾病中存在一种独特实体。
